Daniela Gattini1, Amin J Roberts2, Paul W Wales3, Sue V Beath4, Helen M Evans5, Jonathan Hind6, David Mercer7, Theodoric Wong4, Jason Yap8, Christina Belza9, Koen Huysentruyt1, Yaron Avitzur10. 1. Group for Improvement of Intestinal Function and Treatment, Transplant Centre, Toronto, Ontario, Canada; Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. 2. Group for Improvement of Intestinal Function and Treatment, Transplant Centre, Toronto, Ontario, Canada; Department of Paediatric Gastroenterology, Starship Child Health, University of Auckland, Auckland, New Zealand. 3. Group for Improvement of Intestinal Function and Treatment, Transplant Centre, Toronto, Ontario, Canada; Division of General and Thoracic Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. 4. Department of Gastroenterology and Nutrition, Nutrition Support and Intestinal Failure team, Birmingham Women's and Children's Hospital, Birmingham, United Kingdom. 5. Department of Paediatric Gastroenterology, Starship Child Health, University of Auckland, Auckland, New Zealand. 6. Paediatric Liver, GI, and Nutrition Centre, King's College Hospital, London, United Kingdom. 7. Organ Transplant Center, University of Nebraska Medical Center, Omaha, NE. 8. Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada. 9. Group for Improvement of Intestinal Function and Treatment, Transplant Centre, Toronto, Ontario, Canada. 10. Group for Improvement of Intestinal Function and Treatment, Transplant Centre, Toronto, Ontario, Canada; Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Electronic address: yaron.avitzur@sickkids.ca.
Abstract
OBJECTIVES: To assess the natural history and outcomes of children with intestinal failure in a large, multicenter, geographically diverse contemporary cohort (2010-2015) from 6 pediatric intestinal failure programs. STUDY DESIGN: Retrospective analysis of a multicenter intestinal failure cohort (n = 443). Competing-risk analysis was used to obtain cumulative incidence rates for the primary outcome (enteral autonomy, transplantation, or death). The χ2 test and Cox proportional hazard regression were used for bivariate and multivariable analyses. RESULTS: The study cohort comprised 443 patients (61.2% male). Primary etiologies included short bowel syndrome (SBS), 84.9%; dysmotility disorder, 7.2%; and mucosal enteropathy, 7.9%. Cumulative incidences for enteral autonomy, transplantation, and death at 6 years of follow-up were 53.0%, 16.7%, and 10.5%, respectively. Enteral autonomy was associated with SBS, ≥50% of small bowel length, presence of an ileocecal valve (ICV), absence of portal hypertension, and follow-up in a non-high-volume transplantation center. The composite outcome of transplantation/death was associated with persistent advanced cholestasis and hypoalbuminemia; age <1 year at diagnosis, ICV, and intact colon were protective. CONCLUSIONS: The rates of death and transplantation in children with intestinal failure have decreased; however, the number of children achieving enteral autonomy has not changed significantly, and a larger proportion of patients remain parenteral nutrition dependent. New strategies to achieve enteral autonomy are needed to improve patient outcomes.
OBJECTIVES: To assess the natural history and outcomes of children with intestinal failure in a large, multicenter, geographically diverse contemporary cohort (2010-2015) from 6 pediatric intestinal failure programs. STUDY DESIGN: Retrospective analysis of a multicenter intestinal failure cohort (n = 443). Competing-risk analysis was used to obtain cumulative incidence rates for the primary outcome (enteral autonomy, transplantation, or death). The χ2 test and Cox proportional hazard regression were used for bivariate and multivariable analyses. RESULTS: The study cohort comprised 443 patients (61.2% male). Primary etiologies included short bowel syndrome (SBS), 84.9%; dysmotility disorder, 7.2%; and mucosal enteropathy, 7.9%. Cumulative incidences for enteral autonomy, transplantation, and death at 6 years of follow-up were 53.0%, 16.7%, and 10.5%, respectively. Enteral autonomy was associated with SBS, ≥50% of small bowel length, presence of an ileocecal valve (ICV), absence of portal hypertension, and follow-up in a non-high-volume transplantation center. The composite outcome of transplantation/death was associated with persistent advanced cholestasis and hypoalbuminemia; age <1 year at diagnosis, ICV, and intact colon were protective. CONCLUSIONS: The rates of death and transplantation in children with intestinal failure have decreased; however, the number of children achieving enteral autonomy has not changed significantly, and a larger proportion of patients remain parenteral nutrition dependent. New strategies to achieve enteral autonomy are needed to improve patient outcomes.
Authors: Antonella Lezo; Antonella Diamanti; Evelyne M Marinier; Merit Tabbers; Anat Guz-Mark; Paolo Gandullia; Maria I Spagnuolo; Sue Protheroe; Noel Peretti; Laura Merras-Salmio; Jessie M Hulst; Sanja Kolaček; Looi C Ee; Joanna Lawrence; Jonathan Hind; Lorenzo D'Antiga; Giovanna Verlato; Ieva Pukite; Grazia Di Leo; Tim Vanuytsel; Maryana K Doitchinova-Simeonova; Lars Ellegard; Luisa Masconale; María Maíz-Jiménez; Sheldon C Cooper; Giorgia Brillanti; Elena Nardi; Anna S Sasdelli; Simon Lal; Loris Pironi Journal: Nutrients Date: 2022-04-30 Impact factor: 6.706
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